Retinal Arteriolar Occlusions Research Articles

Protein S Deficiency and Retinal Arteriolar Occlusion in Pregnancy

To report two cases of retinal arteriolar occlusion in pregnant women with transient protein S (PS) deficiency. Observational case reports. Two pregnant women in their 30s presented with a paracentral scotoma in their right and left eye, respectively. In both cases the only risk factor for vascular occlusion was pregnancy. Systemic diseases were excluded. Free and functional PS activity was physiologically reduced in both patients. In Case 1, free PS was 47% and functional PS was 22%. In Case 2, free PS was 43% and functional PS was 25%. These levels of PS seem to be lower than those published for normal pregnancy. There might be a special relationship between PS deficiency and the development of arterial occlusion in pregnant women. A direct effect may be possible. Although the prevalence and incidence of vasoocclusive disease in these patients are low, PS deficiency should be considered as another risk factor. Further studies are necessary to evaluate changes in PS and to assess its relationship with thromboembolic events during pregnancy.

Long-Term Outcome in Susac Syndrome

Susac syndrome is characterized by the clinical triad of encephalopathy, hearing loss, and retinal artery branch occlusions, mostly in young women. To our knowledge, long-term outcome and impact of pregnancy have not been specifically addressed. We report a series of 9 patients (7 female, 2 male) followed at the same institution, with special emphasis on clinical outcome including pregnancy and long-term sequelae. Clinical, brain magnetic resonance imaging (MRI), funduscopy, retinal angiography, and audiogram data were recorded every 3-12 months. We also analyzed the 92 previously reported cases of Susac syndrome. Mean follow-up was 6.4 years. Age at onset was 30.4 years. The first symptom occurred between April and September in 7 of 9 patients in the current study, and in 68% of all patients. The complete triad at onset was clinically obvious in only 1 of 9 patients. Brain involvement was heralded by headache and symptoms of encephalopathy. Cerebrospinal fluid was abnormal in 5 patients showing pleocytosis (mean, 24.6; range, 6-85 cells/mL) and elevated protein level (mean, 210; range, 113-365 mg/dL). Over time, quantitative brain MRI analysis showed that the number of lesions diminished and did not parallel clinical flares, and MRI never normalized. At the end of follow-up, no patient had severe impairment, and all but 1 returned to work. Inner ear involvement was present at onset in 2 patients and occurred in others with a mean delay of 11 months. Initially unilateral in 3, it became bilateral in all. Mean hearing loss was 34 dB (range, 15-70 dB). Hearing loss never improved, either spontaneously or under treatment. The eye was involved at onset in 8 patients, and after 3 years in 1. All had multiple bilateral retinal artery branch occlusions and/or dye leakage with hyperfluorescence of the arterial wall on fluorescein angiography. Over time, angiography normalized in 3 patients. In others, it was still abnormal at the end of follow-up (range, 1.5-10 yr). On late findings, fluorescein leakage was more frequent than true arterial occlusion. Eye involvement was mostly asymptomatic, unilateral, peripheral, and resumed spontaneously to remit in other sites over time. Corticosteroids were efficient to treat encephalopathy, with relapses occurring when the dosage was tapered. Steroid treatment did not improve hearing loss or prevent new retinal arteriolar occlusions. Anticoagulation had a role in treating encephalopathy and retinal arteriolar occlusions. Three patients had 4 pregnancies. Two pregnancies needed induced abortion. One pregnancy was uneventful. One pregnancy was complicated with Susac disease flare in the early postpartum period. In conclusion, at the end of follow-up, most patients had returned to work and none had severe impairment. Pregnancy may affect the course of Susac syndrome, with relapse of encephalopathy postpartum. Our main finding was that the course of Susac syndrome is not self-limited as previously thought, since isolated retinal arteriolar involvement may occur as a very late manifestation.

Susac Syndrome in a Patient With Hepatitis C

A 38-year-old woman seropositive for hepatitis C developed headache, sensorineural hearing loss, encephalopathy, and retinal arteriolar occlusions. Brain MRI showed signal abnormalities in the basal ganglia and corpus callosum. These features are consistent with Susac syndrome, a multifocal central nervous system disorder of uncertain etiology. This is the first reported case of Susac syndrome in a patient with hepatitis C.

Interferon löst okuläre Ischämien aus

Reports of interferon-associated retinopathy mainly originate from Japanese working groups. Only a few cases have been described in Europe. We have performed a retrospective analysis of the patients who were examined in the University Eye Clinic in Mainz complaining of visual loss associated with systemic interferon therapy in the time span between January 2001 and December 2003. 12 patients (age 35 - 67 years), undergoing treatment with interferon alpha for hepatitis C, metastatic renal cell carcinoma, leukaemia and malignant melanoma of the skin, complained of blurred vision 2 - 10 weeks after the start of interferon therapy. Fundus examination revealed retinal haemorrhages and cotton-wool spots in all eyes. Additionally in some eyes macular edema, retinal arteriolar occlusion or anterior ischemic optic neuropathy was seen. As underlying risk factors we found diabetes mellitus (6 x) and systemic hypertension (3 x). After interruption (10 x) or dose reduction (2 x) of interferon therapy in all eyes the ischemic changes disappeared rapidly. Permanent visual loss or visual field defects remained in 3 out of 24 eyes. Patients under interferon therapy should be monitored closely by an ophthalmologist, especially those with systemic vascular risk factors. In the case of development of ischemic microangiopathy of the retina or optic nerve, interferon therapy should be stopped or dose reduction is recommended.

Optical Coherence Tomography Demonstration of Macular Infarction in Sickle Cell Retinopathy

Sickle cell retinopathy is caused by retinal ischemia secondary to the sickling of red blood cells in retinal arterioles, which supply nutrients to the ganglion cell layer, inner nuclear layer, and Muellerian glia of the retina. Macular infarction due to sickle cell disease has been documented using fluorescein angiography, electroretinography, and histopathologic examination. 1–3 However, optical coherence tomography (OCT) of sickle cell retinopathy has, to our knowledge, never previously been reported. We report a case of macular atrophy secondary to retinal arteriolar occlusion in a patient with sickle cell disease, documented by standardresolution OCT and ultrahigh-resolution OCT, a new imaging technology capable of 2- to 3μm resolution in the axial direction 4 compared with 10 μm with standard-resolution OCT. 5

Purtscher-like retinopathy associated with acute pancreatitis

Purtscher-like retinopathy with bilateral loss of vision is a rare and severe complication that may follow acute pancreatitis. The case of a 35-year-old patient with acute alcoholic pancreatitis who developed sudden loss of visual acuity is described. The ophthalmoscopic examination revealed diffuse retinal whitening of the posterior pole with confluent cotton-wool spots. Fluorescein angiogram showed retinal arteriolar occlusion. The findings were compatible with Purtscher-like retinopathy. Computed tomography of the abdomen demonstrated enlarged liver and pancreas with edema and inflammation. The pathogenesis of this form of retinopathy still remains uncertain and there is no specific treatment available.

Multiple branch retinal arteriolar occlusions associated with smallpox vaccination

We describe a rare combination of multiple multiple branch retinal arteriolar occlusions (BRAO) and encephalopathy, which developed in an adult male 10 days after smallpox vaccination. A 53-year-old physician presented with sudden temporal visual field loss in the left eye. Ten days earlier he had received a smallpox vaccination. Hyperbaric oxygen was administered because of suspected BRAO. Examination on day 3 revealed a decrease in visual acuity and loss of visual field. Fundoscopy of the left eye disclosed a retinal infarct in the inferior macula and multiple cotton wool spots over the entire posterior pole. Fluorescein angiography showed delayed arterial filling and multiple non-perfused areas. Magnetic resonance imaging of the brain disclosed white matter focal lesions. Neurological examination was normal. Audiological examination revealed mild hearing loss caused by an old phonic trauma. A combination of intravenous methylprednisolone and immunoglobulins, recommended for the treatment of Susac syndrome, was administered. At the follow-up visit 3 months later, visual acuity in the left eye improved, fundoscopy showed resolution of the cotton wool spots and temporal disc pallor, and the visual field was substantially restored.

Concurrent vs dependent retinal arteriolar occlusion and central vein occlusion

Concurrent vs dependent retinal arteriolar occlusion and central vein occlusion

Retinal arteriolar obstruction with progestogen treatment of threatened abortion.

To describe a case of a retinal arteriolar occlusion associated with progestogen treatment of threatened abortion. Case report. A 30-year-old woman who had received a 2-week course of high dose intramuscular progestogen for a threatened abortion presented with a paracentral scotoma in her right eye and occlusion of a small arteriole adjacent to the macula. There were no major subsequent variations in haemostasis and pre-existing systemic diseases were excluded. Retinal arteriolar occlusion may be associated with high dose progestogen-only therapy in pregnant women.

Multiple retinal arteriolar occlusions associated with coexisting primary antiphospholipid syndrome and factor V Leiden mutation

PURPOSE: To investigate a case of a young woman with both primary antiphospholipid syndrome and factor V Leiden mutation who developed multiple retinal arteriolar occlusions. METHOD: Case report of a 25-year-old woman with history and laboratory tests confirming the diagnosis of both primary antiphospholipid syndrome and factor V Leiden mutation who presented with blurred vision in both eyes. RESULTS: Multiple retinal arteriolar occlusions were observed in both of her eyes. The patient was treated first with heparin and then with warfarin. CONCLUSIONS: Primary antiphospholipid syndrome and factor V Leiden mutation, as well as other forms of thrombophilia, should be considered in the differential diagnosis of unexplained retinal vascular occlusions. The coexistence of several thrombophilic disorders may carry a particularly high risk for thrombotic manifestations.

Ocular manifestations of cat-scratch disease.

Bartonella henselae has only recently been isolated, characterized, and found to be the principal cause of cat-scratch disease (CSD). The availability of specific serologic investigations has allowed the recognition of a spectrum of ocular CSD syndromes that previously were ill defined and considered idiopathic. The primary inoculation complex causing regional lymphadenopathy is represented in the eye by Parinaud's oculoglandular syndrome; B. henselae is the most common cause. Leber's neuroretinitis has been identified for 80 years, and new data suggest that it is commonly a manifestation of CSD; the extent of the association remains to be determined. CSD optic neuritis is also described. The vitreoretinal manifestations include anterior uveitis, vitritis, pars planitis, focal retinal vasculitis, a characteristic retinal white spot syndrome, Bartonella retinitis, branch retinal arteriolar or venular occlusions, focal choroiditis, serous retinal detachments, and peripapillary angiomatous lesions. The pattern of ocular disease in AIDS-associated B. henselae infections is poorly delineated; unusual manifestations include conjunctival and retinal bacillary angiomatosis. The benefit of antimicrobial therapy for CSD in immunocompetent individuals has been difficult to establish, partly because most infections are self limited. Empirically, azithromycin, ciprofloxacin, rifampin, parenteral gentamicin, or trimethoprim-sulfamethoxazole provide the best therapeutic choices to minimize damage to the eye.

Pathogenesis and Neuroprotective Treatment in Purtscher’s Retinopathy

Purtscher’s retinopathy is characterized by sudden visual loss in severely traumatized patients and is associated with multiple areas of superficial retinal whitening located primarily in the posterior pole. Visual outcome in Purtscher’s retinopathy is variable, and there is no well-defined treatment. We report on a patient with immediate blurred vision in the right eye after a traffic accident. Ophthalmoscopy revealed multiple whitish patches scattered over the macular and peripapillary areas in the right eye. Fluorescein angiography showed multifocal retinal arteriolar occlusion in the early phase and staining of the involved retinal vessels and optic nerve head in the late phase. Indocyanine green angiography (ICG) showed rarefaction of choroidal vessels in the peripapillary area of the right eye at early phase. The late phase ICG study revealed multifocal hypofluorescent patches in the macular and peripapillary areas. Megadose steroid therapy was given with good visual response in the first 2 weeks, and the patient’s vision had recovered completely when followed-up 10 months later.

Retinal and choroidal manifestations of cat-scratch disease

Objective The ability to diagnose cat-scratch disease (CSD) has been facilitated greatly by the recent isolation and characterization of Bartonella henselae (formerly genus Rochalimaea) and Afipia felis and by the subsequent development of specific enzyme-linked immunosorbent assay (ELISA) serologic tests. This study will help define the patterns of posterior segment ocular involvement in patients with confirmed CSD. Design The study design is a retrospective case study and literature review. Participants Two consecutive patients with acute visual loss from retinal manifestations of CSD participated. Interventions The diagnosis was confirmed by B. henselae ELISA testing. Patients underwent extensive medical and ophthalmic investigations to exclude other causes of retinal and choroidal disease. Ophthalmic investigation included fluorescein angiography and visual field testing. One patient received antibiotic therapy with cefotaxime, then with ciprofloxacin, and was treated with oral prednisone. The other patient was improving for several weeks before oral doxycycline was given. Main outcome measures The clinical syndromes observed were studied over time using visual acuity, visual field, and clinical findings. Data were collated with cases from the literature. Results Unilateral neuroretinitis and an unusual macular retinitis developed in patient 1, as did bilateral small intraretinal white spots and a unilateral choroidal infiltrate that continued to develop while the patient received antibiotic treatment. Patient 2 had a branch arteriolar occlusion in relation to a perivascular retinal infiltrate and a few small, bilateral, intraretinal white spots. There was gradual resolution with visual improvement while the patient received the antibiotic treatment, although therapeutic efficacy could not be determined. Patient 1 also received oral corticosteroids. A detailed analysis of the literature placed these findings in context. Conclusions An unusual, well-defined retinal opacification with features of both multiple retinal arteriolar occlusions and a low-grade retinitis was described. Several features also may occur in posterior segment CSD, including neuroretinitis, a retinal white spot syndrome, and focal choroiditis.

Reversal of blindness after facial fracture repair by prompt optic nerve decompression

Blindness associated with facial fracture repair is a devastating complication. Fortunately, its occurrence is rare. Orbital compartment syndrome resulting from retrobulbar hemorrhage is the leading cause of visual compromise after facial trauma surgery, with a reported incidence of 0.3% after zygomatic fracture repair.’ Other factors that may contribute to visual loss include direct intraoperative injury to the optic nerve from surgical manipulation, bony fragments, orbital implants, or inferior retinal arteriolar occlusion associated with postoperative orbital swelling.2,3 cial fracture repair in which the vision was salvaged by orbital and optic nerve decompression. Possible contributing factors to the postoperative blindness are discussed.

Complement-induced retinal arteriolar occlusions in the cat.

To develop an animal model of complement-induced retinal vasculopathy and determine whether it resembles Purtscher's retinopathy. Intravenous cobra venom factor was used to achieve intravascular activation of the complement system in cats. After a single bolus of cobra venom factor (75 units/kg), retinal blood flow was monitored at regular intervals by fluorescein angioscopy and angiography. Multiple small retinal arteriolar occlusions were present during the initial fluorescein transit of the immediate postinjection fluorescein study in 12 of 12 animals. Small, rapidly moving gaps in the fluorescein column were seen in two thirds of the animals observed continuously by fluorescein angioscopy. Angiographically, the obstructions were transient, and filling of the associated patches of capillary nonperfusion occurred within 3 minutes. Purtscher's-like ischemic retinal infarcts did not develop in any eye. Histopathologic analysis failed to demonstrate the nature of the transient vascular obstructive lesions, but indirect evidence suggested the possibility of granulocyte aggregates. Intravascular activation of the complement system produces transient microembolic retinal arteriolar occlusions in the cat. Although this model may represent a mild form of Purtscher's retinopathy, factors in addition to complement activation appear necessary to induce ischemic retinal infarcts.

Visual Field Loss Caused by Retinal Vascular Occlusion After Vitrectomy Surgery

To report complete superonasal retinal arterial occlusion with resulting temporal visual field loss after uneventful pars plana vitrectomy with gas-fluid exchange. Case report. Superonasal retinal arterial occlusion was observed 12 days after vitrectomy surgery. Within 10 weeks, the artery reperfused, leaving minimal vascular attenuation and mild optic atrophy. Uneventful vitrectomy surgery can be associated with notable peripheral visual field loss caused by arterial occlusion in the immediate postoperative period. Rapid resorption of the intraocular gas and timely postoperative examination may disclose retinal arteriolar occlusion; otherwise, subsequent reperfusion may leave little evidence of the vascular event.

Idiopathic Recurrent Branch Retinal Arterial Occlusion: Natural History and Laboratory Evaluation

To investigate the long-term visual and systemic prognosis of patients with idiopathic recurrent branch retinal artery occlusions, and to test recent hypotheses regarding possible causes of this syndrome. The authors retrospectively reviewed the medical and photographic records of 16 eligible patients. Each of 15 living patients was interviewed by one of the authors, then underwent follow-up ophthalmic examination, formal visual field testing, and a battery of clinical laboratory tests. Over a mean follow-up of 9 years, only three eyes (9%) lost visual acuity from foveal ischemia, although nine eyes (28%) had central and/or extensive peripheral visual field loss at final examination. Ocular neovascular complications developed in eight eyes (25%). Eight patients (50%) had associated vestibuloauditory and/or transient sensorimotor symptoms, but serious permanent neurologic deficits or recurrent systemic thromboembolic events did not develop. Although most patients had one or more vaso-occlusive risk factors, extensive laboratory testing failed to define the etiology of the arterial occlusions. On long-term follow-up, the visual, neurologic, and systemic prognosis for most patients with idiopathic recurrent branch retinal arterial occlusions remains favorable. Although it is probable that such patients are etiologically heterogeneous, the authors theorize that many have mild or partial manifestations of the microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions.

Vascular occlusion of the retina: an experimental model. I. Leukocyte aggregates.

A new technique was developed to study the pathogenesis of retinal arteriolar occlusion in the pig. Using a catheter with its tip in front of the exit of the ophthalmic artery, autologous microparticles could be injected directly into the retinal arterial system. These microparticles were C5a-des-Arg stimulated leukocytes, which were aggregated to pellets of different sizes ranging from 0.26 to 1.0 mm. For better adhesion of the aggregates to the endothelium, endothelial damage was induced by the injection of additional substances, including methomidate and endothelial antibodies. In a further series of experiments systemic hypoxemic conditions were created over several hours prior to injection of the leukocyte aggregates. This resulted in cotton-wool spots and arterial branch occlusions with retinal edema. Furthermore, retinal hemorrhages occurred. This experimental model seems to be appropriate for mimicking retinal arteriolar occlusion syndromes.

Vascular occlusion of the retina--an experimental model. II. Platelet aggregates.

Using a new technique to mimic arteriolar occlusion syndromes, different microparticles were injected into the ophthalmic artery of pigs. These microparticles were platelet aggregates of various sizes ranging from 0.15 mm to more than 0.8 mm. Aggregation was induced using adenosine diphosphate and thrombin. In some experiments, acetylsalicylic acid and histamine were also applied. In a further series of experiments, mixed aggregates containing platelets and leukocytes were injected. The results were observed by indirect ophthalmoscopy and consisted of superficial and deep retinal infarction. Large platelet aggregates resulted in arterial branch occlusion, whereas mixed and small platelet aggregates produced occlusion of small arterioles. This technique seems to be valuable in studying retinal arteriolar occlusion syndromes.

Microangiopathy of the brain, retina, and ear: improvement without immunosuppressive therapy.

We describe a pregnant young woman with branch retinal arteriolar occlusions, encephalopathy, and hearing loss in whom we demonstrated a patent foramen ovale. She improved while receiving anticoagulants and no immunosuppressive therapy. The microangiopathic syndrome of retinopathy, encephalopathy, and deafness may be due to a disturbance of coagulation and/or microembolism.